Biobank for Cholestatic Liver Diseases.

This study is a biobank of specimens and clinical data for use in current and future research
to better understand the cholestatic liver diseases primary biliary cirrhosis/cholangitis
(PBC) and primary sclerosing cholangitis (PSC).

Blood, urine, and stool samples will be collected periodically during patient visits or via a
mail-home kit. Bile and bile duct cells will be collected during clinically necessary
Endoscopic Retrograde Cholangiopancreatography (ERCP) procedures. Anticipated research will
focus on multi-omics assessments of biospecimens to better define how these diseases start
and progress in order to develop novel tests for early detection of complication and better
disease prognostication.

Inclusion Criteria:

PSC

- Patients diagnosed with PSC and who are between the age of 18 and 85 at time of
enrollment in the study.

- The diagnosis of PSC will be based on standard PSC criteria including clinical and
biochemical evidence of chronic cholestasis of at least six months duration, positive
cholangiographic findings, and compatible liver biopsies if available.

- Patients with PSC who have undergone orthotopic liver transplantation will be offered
enrollment except for collection of bile - please see below under Exclusion Criteria.

- Women with PSC of childbearing potential and pregnant women will be offered enrollment
because there is no risk to an unborn child in this investigation.

PBC

- Patients diagnosed with PBC and who are between the age of 18 and 85 at time of
enrollment in the study.

- The diagnosis of PBC will be based on standard PBC criteria including clinical and
biochemical evidence of chronic cholestasis of at least six month duration, positive
anti-mitochondrial antibodies in serum and compatible liver biopsies, if available.

- Patients with PBC who have undergone orthotopic liver transplantation will be offered
enrollment except for collection of bile - please see below under Exclusion Criteria.

- Women with PBC of childbearing potential and pregnant women will be offered enrollment
because there is no risk to an unborn child in this investigation.

Controls

- Controls without history of PBC, PSC, or evidence of other chronic liver disease of
either gender that participate in this study will be between the ages of 18 and 85.

Liver Disease Controls

- Patients without history of PBC or PSC but do have evidence of other chronic liver
disease of either gender will be offered participation in this study if between the
ages of 18 and 85.

Exclusion Criteria (all subjects):

- PBC or PSC patients with known and overlapping other chronic liver diseases

- Patients unable to provide informed consent

- Prisoners and institutionalized individuals

Exclusion Criteria (for bile collection during ERCP)

- PSC with orthotopic liver transplantation

- History of chemotherapy treatment